INTRODUCTION
Intrahepatic interruption of the inferior vena cava (IVC) with azygos vein continuation is a rare congenital aberration, occurring in 0.6% of patients with other cardiac defects as situs abnormalities and dextrocardia.­1 In most instances, patients are asymptomatic. They might be noticed as incidental findings following imaging investigations. We present a case of situs inversus totalis and dextrocardia associated with interrupted inferior vena cava (IVC) and azygos vein continuation in a patient with concomitant long-persistent atrial fibrillation. The treatment of atrial fibrillation (AF) has been considered at high risk for percutaneous interventions and thus referred to our institute for surgical bilateral thoracoscopic epicardial ablation and left atrial appendage (LAA) exclusion.
The patient is a 45-years old male with symptomatic long-standing persistent atrial fibrillation (LsP-AF) who received multiple electrical cardioversions for AF recurrences since 2003. Class I antiarrhythmic drugs failed to restore sinus rhythm thus, according to the current ESC 2020 Guidelines for AF treatment 2, the patient was scheduled for transcatheter pulmonary veins (PV) isolation.3Pre-operative chest X-RAY showed the presence of a complete situs inversus dextrocardia (SID). (Figure 1A)
Then, a thoraco-abdominal CT scan was performed to rule out other anatomical abnormalities. A concomitant intrahepatic interruption of the IVC was described with the renal veins draining into the azygos vein which was directly collecting blood from the lower body and draining posteriorly into the superior vena cava (SVC). In adjunct, the hepatic veins were draining directly into the right atrium (RA). Nonetheless, regular anatomy of the left atrium (LA) with two right and two left PVs was depicted (Figure 1B, 1C).
The patient was deemed not suitable for percutaneous ablation (PA) because of the complex anatomy and was then referred to our Institution for surgical thoracoscopic ablation. Transesophageal echocardiogram was performed to rule out thrombus in the LAA prior to surgery.